Congenital heart disease (CHD) is an important subset of all cardiovascular diseases in dogs. Among them, there are a number of other less commonly described congenital malformations. One of these is the atrioventricular canal, which involves a defect that has several phenotypes. This report details a case of a complete atrioventricular canal (cAVC) defect. Using Rastelli's classification scheme, the diagnosis of a type-A cAVC defect was based on two-dimensional, contrast, and color Doppler echocardiography. Despite a severe defect that resulted in the atypical anatomy of the atrioventricular apparatus, as well as significant hemodynamic changes in the heart, the affected dog remained asymptomatic throughout the 48-month follow-up period. Due to its stable clinical condition, the decision was made not to begin pharmacological therapy. As this defect is rare in dogs, there are limited data in the literature on this condition. Therefore, in clinical practice, the management and monitoring of the patient may pose difficulties. Due to such limited data, it can be very difficult to provide a prognosis. For the above reasons, we hope that the following case will contribute valuable information for the monitoring of this kind of CHD.